Effective pulmonary host defense is orchestrated by complex interactions between
pulmonary surfactant and alveolar macrophages. Pulmonary macrophages fulfill diverse roles in
surveillance and inactivation of inhaled pathogens and in regulating surfactant homeostasis.
Pulmonary surfactant proteins have innate protective activity and influence macrophage
function. Mutations in genes that alter surfactant proteins, surfactant production or catabolism
in the lung have been linked to human disease and are associated with abnormal macrophage
function or alternative activation of pulmonary macrophages. Manipulation of the analogous
genes in transgenic and gene targeted mice replicate aspects of macrophage related pulmonary
disease. The genetic mouse models have been used to investigate the molecular pathogenesis
and progression of acute and chronic lung disease of infancy, familial interstitial lung disease
and pulmonary alveolar proteinosis.
